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Rapid enlargement of a syringomyelia cavity following syringo-subarachnoid shunt: case report.

We describe a patient who presented with syringomyelia 20 years following an operation for foramen magnum meningioma. A syringo-subarachnoid shunt was placed, which connected the syrinx and the cerebello-pontine angle cistern. Following shunt placement, the patient's symptoms rapidly progressed, and magnetic resonance imaging (MRI) revealed enlargement of the syrinx. The symptoms improved with a decrease in syrinx size after placement of a syringo-peritoneal shunt in combination with revision of the syringo-subarachnoid shunt, changing the position of the subarachnoid tip to the caudal spinal subarachnoid space. The mechanism of rapid enlargement of the syrinx cavity following placement of the syringo-subarachnoid shunt is discussed.

Aged↗

Syringomyelia: myelography, computed tomography, and magnetic resonance imaging.

Seventeen patients with cervical spinal cord cavities were studied with myelography, postmyelographic computed tomography scanning, and magnetic resonance imaging. The three diagnostic techniques were compared for accuracy, patient comfort, and ease of procedure. Magnetic resonance imaging was the best diagnostic and most comfortable procedure. There is no need for myelography or postmyelographic computed tomography scanning for the evaluation of cervical syringomyelia.

Humans↗

Absence of spinal N13-P13 and normal scalp far-field P14 in a patient with syringomyelia.

Short latency somatosensory evoked potentials to median or ulnar nerve stimulation were recorded in a patient with syringomyelia. Scalp-recorded far-field P14 was clearly preserved, but spinal N13-P13 components disappeared. Our findings support the hypothesis that spinal N13-P13 is generated by structures intrinsic to the cervical cord, most likely in the ventral central gray matter.

Adult↗

Discrepancy between SEPs directly recorded from the dorsal column nuclei following upper and lower limb stimulation in patients with syringomyelia.

Somatosensory evoked potentials (SEPs) in response to electrical stimulation of the median nerve (MN) and posterior tibial nerve (PTN) were studied in 2 patients with syringomyelia. Intraoperative recordings were made from the surface of the dorsal column nuclei as well as from the scalp. Following MN stimulation, there was a preservation of scalp-recorded P9, P11, P13, and N20, however, there was an absence of spinal N13-P13. The dorsal column SEPs to MN stimulation were normal, characterized by a major negativity (N1), preceded by a small positivity (P1) and followed by a large positivity (P2). On the other hand, there was little or no cortical response (P37) to PTN stimulation. The dorsal column SEPs to PTN stimulation showed a disappearance of the normal P1'-N1'-P2' configuration, being replaced by a series of small spiky waves. The syringomyelic cavity may have thus compressed the gracile dorsal column which courses more medially than the cuneate pathway, causing desynchronization of the dorsal column SEPs. These findings suggest that dorsal column pathway arising from the lower limb is more vulnerable than that from the upper limb when a cervical syrinx is present.

Adult↗

Evaluation of syringomyelia and Chiari malformations using ultra-low magnetic resonance imaging.

The accuracy of ultra-low field magnetic resonance imaging (MRI) compared to medium-field MRI in detecting syringomyelia and associated Chiari malformations is examined. In all cases the size of the syrinxes and the type and extent of the Chiari malformations was clearly shown at low-field MRI. The present spatial solution and high sensitivity allows for low-field imagers to localize even minor morphological changes of the spinal cord, brainstem, and cerebellum.

Adolescent↗

Congenital muscular dystrophy with syringomyelia.

We report a 7-year-old boy with congenital muscular dystrophy with severe spinal deformation and low thoracic syringomyelia, which may represent a novel form of the disease with muscle involvement and spinal cord anomaly. We suggest that patients with congenital muscular dystrophy who manifest skeletal anomalies undergo spinal magnetic resonance imaging to detect potential spinal cord abnormalities, in addition to cranial magnetic resonance imaging to detect potential cerebral malformations.

Biopsy↗

Isolated sleep apnea due to Chiari type I malformation and syringomyelia.

We report an 11-year-old girl with Chiari type I malformation and syringomyelia, who experienced isolated sleep apnea without other neurologic problems. Monitoring with oximetry and movement of thoracic and abdominal walls indicated mixed-type sleep apnea. Chiari type I malformation should be differentiated from other disorders causing sleep apnea.

Arnold-Chiari Malformation↗

Surgical experience of syringomyelia with reference to the findings of magnetic resonance imaging.

We present our surgical experience of 20 patients with syringomyelia, who were divided into two groups based on the findings of magnetic resonance (MR) imaging: a "non-visible cisterna magna" group, in which MR imaging did not reveal cerebrospinal fluid (CSF) in the cisterna magna, and a "visible cisterna magna" group. Patients with non-visible cisterna magna were associated with Chiari malformation (14 patients) or tight cisterna magna (4 patients) and underwent craniocervical decompression. Intradural exploration was performed when CSF movement in the cisterna magna or CSF outflow from the fourth ventricle appeared to be insufficient. It is important to confirm CSF outflow from the foramen of Magendie. Patients with visible cisterna magna were associated with tuberculous meningitis (2 patients) and underwent shunting procedures. Postoperatively, improvement in symptoms and a reduction in syrinx size were demonstrated in all patients except one. Two patients experienced recurrence of symptoms and syrinx dilatation.

Adolescent↗

A modified technique for syringo-subarachnoid shunt for treatment of syringomyelia.

Syringo-subarachnoid shunting is a well-established procedure for the treatment of syringomyelia. However, the standard surgical procedure requires a laminectomy and posterior midline myelotomy, which have potential complications. In this study, we describe our clinical experience with a modified technique for syringo-subarachnoid shunt insertion in eight patients between 1998 and 2002. The technique comprises a limited hemilaminectomy, a 2 mm myelotomy at the site of dorsal root entry zone, introduction of a 1.5 mm thick catheter into the syrinx and placement of the distal tip of the catheter in the anterolateral subarachnoid space. Using this technique there was no operative morbidity or mortality. Collapse of the syrinx, in the first post-operative month, was demonstrated by MRI in all cases. There were no relapses in the follow-up period. Although our experience is limited and the results preliminary, this technique is less invasive than commonly used techniques and the results are favorable.

Adult↗

Spontaneous chronic spinal subdural hematoma associated with spinal arachnoiditis and syringomyelia.

Spontaneous chronic spinal subdural hematoma is rare. We describe a case of spontaneous chronic spinal subdural hematoma associated with arachnoiditis and syringomyelia in a 76-year old woman who presented with a 14-year history of progressive myelopathy. MRI scan revealed a thoraco-lumbar subdural cystic lesion and a thoracic syrinx. The patient underwent thoracic laminectomy and decompression of the lesion, which was a subdural hematoma. A myelotomy was performed to drain the syrinx. Pathological examination revealed features consistent with chronic subdural membrane. This report attempts to elucidate the pathogenesis of chronic spinal subdural hematoma. We discuss possible etiological factors in light of the current literature and pathogenesis of both spinal subdural hematoma and syrinx formation.

Aged↗

Surgical treatment of a syringomyelia associated with an idiopathic arachnoid malformation disclosed by preoperative MRI.

BACKGROUND: We describe the very rare condition of an idiopathic spinal arachnoid malformation associated with syringomyelia (SM) and depicted on preoperative magnetic resonance imaging (MRI) whose features were confirmed at surgery. CASE DESCRIPTION: A 34-year-old female suffered from progressive gait impairment because of lower limb palsy and sensory disturbances. MRI demonstrated a bulging membrane at the T6 level that was transversely stretched between the dorsal aspect of the spinal cord and the posterior dura mater. At this level, the spinal cord appeared atrophic and pushed anteriorly against the dura with enlargement of the posterior subarachnoid spaces (SAS) and focal collapse of an associated panmedullar SM. Surgery consisted in releasing the arachnoid malformation and opening the inferior segment of the syringomyelic cavity. Pathological examination revealed a fibro-sclerotic tissue with cellular areas of meningo-endothelial cells. Postoperative neurological status progressively improved but slightly. Three-months and 1 year postoperatively, MRI showed the collapse of the whole SM and restoration of cerebrospinal fluid (CSF) flow at the treated T6 level. CONCLUSION: Spinal arachnoid malformations associated with SM are very rare and have never been described up to now on MRI. Surgical removal of the causative malformation allows spinal cord decompression and prevents the recurrence of the SM by restoring normal CSF circulation.

Adult↗

Neurosurgical considerations in posttraumatic syringomyelia.

Syringomyelia is a progressive degenerative disorder that results in numerous life-altering symptoms. People with this disorder frequently are misdiagnosed and most likely will experience intractable pain for many years. Surgical procedures may offer some relief from symptoms through spinal decompression or cerebrospinal fluid shunting; however, these procedures often are unsuccessful in altering the course or progression of the disease process. Information on the implications of the disease offers insight that is helpful for nurses providing care to these patients. This article presents relevant clinical information and discusses surgical procedures, as well as a patient's perspective on living with this relatively unknown disorder.

Adult↗

Multiple sclerosis with secondary syringomyelia. An autopsy report.

We report an elderly woman with multiple sclerosis who showed an extensive cavity formation in the midthoracic cord in addition to multiple abnormal intensity signals in the central nervous system on magnetic resonance imaging (MRI). The cavity decreased in size in response to corticosteroid therapy with an improvement in neurological symptoms. The autopsy demonstrated a slit-like cavity lined with no ependymal cells on the luminal surface in the lower cervical to midthoracic cord, with circumferentially distributed demyelinative lesions, leading to the pathological diagnosis of secondary syringomyelia. In this patient a limited necrosis formed in the spinal cord might have developed into a cavity formation with edematous fluid leading to subsequent episodes of neurological exacerbation.

Aged↗

[Spontaneous resolution of syringomyelia in a child with Chiari I malformation: a case report].

Cervicothoracic syringomyelia is a frequent feature in Chiari I malformation. It can be symptomatic or not, and is well demonstrated by magnetic resonance imaging (MRI). Its spontaneous resolution is uncommon. The authors report a case of spontaneous resolution of a thoracic syrinx in an 18-year-old patient with a Chiari I malformation. MRI study performed 6 years previously because of worsening headaches demonstrated a Chiari I malformation associated with a syrinx cavity. The cavity disappearance was noted after improvement of the symptoms.

Adolescent↗

Syringomyelia and pregnancy-case report.

The course of a pregnancy in a woman with syringomyelia is presented. She was first admitted at 28 weeks' gestation suffering neurologic symptoms associated with a spinal cord injury, which had happened in the past. The disease was diagnosed with a magnetic resonance imaging (MRI). Delivery was accomplished by elective caesarean section under general anaesthesia at 37 weeks, in order to avoid straining during the second stage of an imminent labour.

Cesarean Section↗

Posterior fossa decompression in syringomyelia associated with a Chiari malformation: a retrospective analysis of 22 patients.

BACKGROUND: The technique of posterior fossa decompression, nowadays preferred by most neurosurgeons as the first surgical option in the treatment of hindbrain associated syringomyelia, was reintroduced in our center in 1989. We present a retrospective analysis of 22 patients with this pathology who underwent the procedure since then. METHODS: In all patients diagnosis was made by MRI of the craniospinal junction. The operation consisted of a suboccipital craniectomy, removal of the posterior arc of C1, laminectomy of C2 or C3 when necessary and a wide duraplasty at the end. The intradural manipulations (opening of the arachnoid membrane, coagulation or resection of the tonsils) were not uniform. Postoperatively short- and long-term clinical outcome and MRI findings were assessed. RESULTS: Sixteen out of 21 patients (76%) experienced an improvement in the early follow-up period. In the late follow-up period 13 out of 19 patients (68%) were improved, whereas five patients (26%) experienced a marked deterioration. There was no unequivocal effect on all symptoms and signs. Postoperative MR images showed a favorable result in 16 out of 20 patients (80%), consisting of syrinx collapse or reduction of the syrinx diameter. CONCLUSIONS: We conclude that decompression of the posterior fossa is a safe procedure with a considerable chance of clinical improvement. Although total syrinx collapse is not as frequently seen as in syrinx shunting procedures, the clinical outcome may be better. Moreover, there seemed to be no unequivocal correlation between clinical outcome and postoperative syrinx size in the present study.

Adolescent↗

Post-traumatic syringomyelia: a review.

More than a quarter of spinal cord injured patients develop syringes and many of these patients suffer progressive neurological deficits as a result of cyst enlargement. The mechanism of initial cyst formation and progressive enlargement are unknown, although arachnoiditis and persisting cord compression with disturbance of cerebrospinal fluid flow appear to be important aetiological factors. Current treatment options include correction of bony deformity, decompression of the spinal cord, division of adhesions, and shunting. Long-term improvement occurs in fewer than half of patients treated. Imaging evidence of a reduction in syrinx size following treatment does not guarantee symptomatic resolution or even prevention of further neurological loss. A better understanding of the causal mechanisms of syringomyelia is required to develop more effective therapy.

Accidents↗